Urinary oligosaccharides of GM1-gangliosidosis. Structures of oligosaccharides excreted in the urine of type 1 but not in the urine of type 2 patients.
Among the seven oligosaccharide fractions obtained by Bio-Gel P-4 column chromatography of urine of GM1-gangliosidosis Type 1 patients, three fractions (peaks V, VI, and VII) were completely missing in the urine of GM1-gangliosidosis Type 2 patients. Structural study of these oligosaccharide fractions by sequential exoglycosidase digestion in combination with methylation analysis and periodate oxidation has shown that peaks V, VI, and VII are mixtures of 16, 30, and 49 isomeric oligosaccharides. All these 95 oligosaccharides contain Gal beta 1 leads to 4GlcNAc beta 1 lead to 3 repeating structures in their outer chain moieties, indicating that the tissues of GM1-gangliosidosis Type 2 patients do contain beta-galactosidase activity which releases readily galactose residue from such repeating sugar chains.
D002236:Carbohydrate Conformation; D002240:Carbohydrate Sequence; D005677:G(M1) Ganglioside / Q000378:metabolism*; D005732:Gangliosides / Q000378:metabolism*; D005733:Gangliosidoses / Q000652:urine*; D006026:Glycoside Hydrolases; D006801:Humans; D008745:Methylation; D009844:Oligosaccharides / Q000652:urine*
D005732:Gangliosides; D009844:Oligosaccharides; D005677:G(M1) Ganglioside; D006026:Glycoside Hydrolases